Clinical characteristics of pancreatic and biliary tract cancers associated with Lynch syndrome

Abstract

BACKGROUND/PURPOSE: Pancreatic and biliary tract cancers are one of the Lynch syndrome-associated malignancies. There are few reports describing the patients' backgrounds and clinical characteristics. METHODS: We retrospectively reviewed the medical records of patients with Lynch syndrome-associated pancreatic or biliary tract malignant tumors at National Cancer Center Hospital between March 1992 and October 2019. RESULTS: Fourteen patients were included. They had a history of multiple cancers and a family history of cancer. For the six patients with pancreatic malignant tumor, the median age was 63 years. The primary tumor site of 5/6 patients with pancreatic cancer was the body or tail. Only one patient had pancreatic head cancer. The median overall survival (OS) was 68 (range, 17-198) months. For the eight patients with biliary tract malignant tumor, the median age was 65.5 years. The primary tumor site of 5/8 patients was the intrahepatic bile duct, whereas the primary site of 2/8 was the hilar bile duct. The median OS was 62 (range, 3-183) months. CONCLUSIONS: This study brought out several observations on tumor location, late development, and favorable long-term outcomes. Additional studies are needed to identify the characteristics.